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https://www.readbyqxmd.com/read/29455235/enlarged-spleen-is-associated-with-low-neutrophil-and-platelet-engraftment-rates-and-poor-survival-after-allogeneic-stem-cell-transplantation-in-patients-with-acute-myeloid-leukemia-and-myelodysplastic-syndrome
#1
Yoshimitsu Shimomura, Masahiko Hara, Daisuke Katoh, Hisako Hashimoto, Takayuki Ishikawa
Primary graft failure can be a cause of early morbidity and mortality after allogeneic hematopoietic stem cell transplantation (HSCT), as it leads to a high risk of severe infections and bleeding. Splenomegaly is associated with primary graft failure in patients of myelofibrosis, but the association between splenomegaly and outcomes after HSCT in patients with myeloid malignancies has not been previously evaluated. The aim of this study was to investigate the effect of spleen volume on engraftment kinetics in patients with acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS)...
February 17, 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29455198/incidence-survival-and-risk-factors-for-adults-with-acute-myeloid-leukemia-not-otherwise-specified-and-acute-myeloid-leukemia-with-recurrent-genetic-abnormalities-analysis-of-the-surveillance-epidemiology-and-end-results-seer-database-2001-2013
#2
Xiaolu Song, Ye Peng, Xiaogang Wang, Yirui Chen, Lai Jin, Tianxin Yang, Meihua Qian, Wanmao Ni, Xiangmin Tong, Jianping Lan
BACKGROUND/AIM: As the knowledgebase of acute myeloid leukemia (AML) has grown, classification systems have moved to incorporate these new findings. METHODS: We assessed 32,941 patients with AML whose records are contained in the Surveillance, Epidemiology, and End Results (SEER) database. RESULTS: Half of all patients diagnosed between 2001 and 2013 did not have a World Health Organization (WHO) classification. Acute promyelocytic leukemia and acute panmyelosis with myelofibrosis were associated with the longest leukemia-specific survival (110 and 115 months, respectively), and AML with minimal differentiation and acute megakaryoblastic leukemia with the shortest (30 and 28 months, respectively)...
February 16, 2018: Acta Haematologica
https://www.readbyqxmd.com/read/29428448/pulmonary-langerhans-cell-histiocytosis-acute-myeloid-leukemia-and-myelofibrosis-in-a-large-family-and-review-of-the-literature
#3
Matthew P Blakley, Janice P Dutcher, Peter H Wiernik
BACKGROUND: There is mounting evidence that Langerhans cell histiocytosis (LCH) and acute myeloid leukemia (AML) are hematopoietic neoplasms that arise from the same myeloid precursor cell. In addition, studies suggest a relationship between LCH and primary idiopathic myelofibrosis (MF). Furthermore familial LCH, AML, and MF have each been reported. METHODS: We examined more than 750 pedigrees of familial hematologic malignancies for evidence of familial LCH, AML, and/or MF and identified one family with all three neoplasms, which is presented here...
February 2, 2018: Leukemia Research
https://www.readbyqxmd.com/read/29427319/distinguishing-myelofibrosis-from-polycythemia-vera-and-essential-thrombocythemia-the-utility-of-enumerating-circulating-stem-cells-with-aberrant-hmicl-expression-by-flow-cytometry
#4
L L Herborg, L Nederby, H C Hasselbalch, A Aggerholm, A S Roug
INTRODUCTION: Diagnosing BCR-ABL negative myeloproliferative neoplasms (MPN) may be challenging due to overlapping features and lack of robust discriminatory parameters, especially between essential thrombocythemia (ET) and prefibrotic myelofibrosis (MF). Circulating immature hematopoietic cells are variably present in polycythemia vera (PV), ET, and MF. The C-type lectin hMICL is aberrantly expressed on hematopoietic stem cells in the majority of acute myeloid leukemia patients. However, the hMICL expression in MPN, having varying propensity of leukemic transformation, is unsettled...
February 10, 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29426921/the-2016-who-classification-and-diagnostic-criteria-for-myeloproliferative-neoplasms-document-summary-and-in-depth-discussion
#5
REVIEW
Tiziano Barbui, Jürgen Thiele, Heinz Gisslinger, Hans Michael Kvasnicka, Alessandro M Vannucchi, Paola Guglielmelli, Attilio Orazi, Ayalew Tefferi
The new edition of the 2016 World Health Organization (WHO) classification system for tumors of the hematopoietic and lymphoid tissues was published in September 2017. Under the category of myeloproliferative neoplasms (MPNs), the revised document includes seven subcategories: chronic myeloid leukemia, chronic neutrophilic leukemia, polycythemia vera (PV), primary myelofibrosis (PMF), essential thrombocythemia (ET), chronic eosinophilic leukemia-not otherwise specified and MPN, unclassifiable (MPN-U); of note, mastocytosis is no longer classified under the MPN category...
February 9, 2018: Blood Cancer Journal
https://www.readbyqxmd.com/read/29411299/a-rare-calr-variant-mutation-and-a-review-of-calr-in-essential-thrombocythemia
#6
Robert Diep, Ara Metjian
Essential thrombocythemia (ET) is an indolent myeloproliferative neoplasm characterized by megakaryocyte hyperplasia, thrombocytosis, thrombotic and hemorrhagic complications, and potential transformation into myelofibrosis and acute myeloid leukemia. The vast majority of cases are driven by a somatic mutation in JAK2, CALR, or MPL. CALR, a gene that codes for the calcium-binding chaperone calreticulin, is the predominant mutation in patients with non-mutated JAK2 essential thrombocythemia, accounting for 20-25% of the overall somatic mutation frequency in ET...
February 6, 2018: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/29407589/prognostic-implications-of-low-transferrin-saturation-in-patients-with-primary-myelofibrosis
#7
Marko Lucijanic, Zeljko Prka, Vlatko Pejsa, Tajana Stoos-Veic, Jelena Lucijanic, Rajko Kusec
OBJECTIVES: Transferrin saturation (TSAT) 20% or less is considered to represent functional iron deficiency in the context of malignant disease, phenomenon mediated through inflammatory changes of iron homeostasis. We aimed to investigate clinical and prognostic significance of low TSAT in patients with primary (PMF) and secondary myelofibrosis (SMF), malignant diseases characterized by strong inflammatory milieu. METHODS: We retrospectively analyzed 87 patients with myelofibrosis and compared TSAT with disease specific parameters...
January 29, 2018: Leukemia Research
https://www.readbyqxmd.com/read/29407586/8q24-myc-rearrangement-is-a-recurrent-cytogenetic-abnormality-in-blastic-plasmacytoid-dendritic-cell-neoplasms
#8
Prajwal C Boddu, Sa A Wang, Naveen Pemmaraju, Zhenya Tang, Shimin Hu, Shaoying Li, Jie Xu, L Jeffrey Medeiros, Guilin Tang
8q24/MYC rearrangements resulting in MYC overexpression occur most frequently in lymphoid neoplasms. MYC rearrangements rarely have been described in blastic plasmacytoid dendritic cell neoplasm (BPDCN). Over an 8-year period in our hospital, 5 of 41 (12%) patients with BPDCN were shown 8q24/MYC rearrangements, including 2 with t(6;8)(p21;q24), 1 with t(8;14)(q24;q32), 1 with t(X;8)(q24;q24), and 1 with t(3;8)(p25;q24). 8q24/MYC rearrangement was present in the stemline in 4 patients and in the sideline in one; the latter was a patient with primary myelofibrosis who then developed BPDCN...
February 2, 2018: Leukemia Research
https://www.readbyqxmd.com/read/29405428/the-2014-bcsh-criteria-and-the-2016-who-criteria-for-essential-thrombocythemia-a-comparison-in-a-large-scale-cohort
#9
Tomonori Ochiai, Hajime Yasuda, Marito Araki, Kyohei Misawa, Soji Morishita, Mai Nudejima, Yumi Hironaka, Shuichi Shirane, Yoko Edahiro, Akihiko Gotoh, Akimichi Ohsaka, Norio Komatsu
OBJECTIVE: There are currently two representative diagnostic criteria for essential thrombocythemia (ET), the 2014 British Committee for Standards in Hematology Guidelines (BCSH) criteria and the 2016 World Health Organization (WHO) criteria. We compare and discuss the advantages and disadvantages of the two criteria. METHOD: We applied the two criteria to 403 patients with thrombocytosis and suspected myeloproliferative neoplasms (MPN) and compared patient populations...
February 5, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29403325/tafro-syndrome-current-perspectives
#10
REVIEW
Kentaro Sakashita, Kengo Murata, Mikio Takamori
Multicentric Castleman's disease (MCD), a distinct subtype of Castleman's disease, is a rare, nonneoplastic, lymphoproliferative disorder. Patients with MCD present with systemic symptoms and multiple lymphadenopathy. Lymph node biopsy is necessary for the diagnosis of various histological MCD patterns including hyaline vascular, plasma cell, and mixed types. Human herpesvirus 8 (HHV8) infection was identified as an important etiology of MCD among immunocompromised patients such as those positive for human immunodeficiency virus...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/29400094/gata1-insufficiencies-in-primary-myelofibrosis-and-other-hematopoietic-disorders-consequences-for-therapy
#11
Te Ling, John D Crispino, Maria Zingariello, Fabrizio Martelli, Anna Rita Migliaccio
GATA1, the founding member of a family of transcription factors, plays important roles in the development of hematopoietic cells of several lineages. Although loss of GATA1 has been known to impair hematopoiesis in animal models for nearly 25 years, the link between GATA1 defects and human blood diseases has only recently been realized. Areas covered: Here the current understanding of the functions of GATA1 in normal hematopoiesis and how it is altered in disease is reviewed. GATA1 is indispensable mainly for erythroid and megakaryocyte differentiation...
February 5, 2018: Expert Review of Hematology
https://www.readbyqxmd.com/read/29399328/jak-inhibitors-for-the-treatment-of-myeloproliferative-neoplasms-and-other-disorders
#12
REVIEW
William Vainchenker, Emilie Leroy, Laure Gilles, Caroline Marty, Isabelle Plo, Stefan N Constantinescu
JAK inhibitors have been developed following the discovery of the JAK2 V617F in 2005 as the driver mutation of the majority of non- BCR-ABL1 myeloproliferative neoplasms (MPNs). Subsequently, the search for JAK2 inhibitors continued with the discovery that the other driver mutations ( CALR and MPL ) also exhibited persistent JAK2 activation. Several type I ATP-competitive JAK inhibitors with different specificities were assessed in clinical trials and exhibited minimal hematologic toxicity. Interestingly, these JAK inhibitors display potent anti-inflammatory activity...
2018: F1000Research
https://www.readbyqxmd.com/read/29397859/-research-progress-on-effect-of-jak-inhibitors-on-myelofibrosis-review
#13
Jun-Xiu Liu, Wei Chen, Kai-Lin Xu
Myelofibrosis(MF) is a type of myeloprolifirative neoplasms which is difficult to be treated. With the discovery of V617F mutation site in Janus kinase 2 (JAK2), JAK inhibitor provides a new treatment strategy for patients with myelofibrosis. Since 2011 the FDA in USA approved the first generation of JAK inhibitor Ruxolitinib for marketing, a growing number of JAK inhibitors have been entering into the clinical trials and showed a certain clinical efficacy. On the one hand, some JAK inhibitors for single application can effectively relieve the clinical symptoms of patients with myelofibrosis, slow down disease progression, and prolong the survival; on the other hand, JAK inhibitor can also be applied in combination with traditional or other new targeted drugs for MF patients, even during the allogenetic hematopoietic stem cell transplantation, thus providing more choices for targeted therapy on the patients with myelofibrosis...
February 2018: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/29396714/prognostic-risk-models-for-transplant-decision-making-in-myelofibrosis
#14
Juan-Carlos Hernández-Boluda, Arturo Pereira, Juan-Gonzalo Correa, Alberto Alvarez-Larrán, Francisca Ferrer-Marín, José-María Raya, Joaquín Martínez-López, Patricia Velez, Manuel Pérez-Encinas, Natalia Estrada, Valentín García-Gutiérrez, María-Laura Fox, Angel Payer, Ana Kerguelen, Beatriz Cuevas, María-Antonia Durán, María-José Ramírez, María-Teresa Gómez-Casares, María-Isabel Mata-Vázquez, Elvira Mora, Montse Gómez, Francisco Cervantes
Prognostic models are widely used in clinical practice for transplant decision-making in myelofibrosis (MF). We have compared the performance of the International Prognostic Scoring System (IPSS), dynamic IPSS (DIPSS), and DIPSS-plus in a series of 544 patients with primary or secondary MF aged ≤ 70 years at the time of diagnosis. The median projected survival of the overall series was 9.46 years (95% confidence interval 7.44-10.59). Median survival for the highest risk groups was less than 4 years in the three prognostic models...
January 25, 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29395255/lessons-learned-from-bone-marrow-failure-in-systemic-lupus-erythematosus-case-reports-and-review-of-the-literature
#15
Erik Anderson, Bhakti Shah, Anne Davidson, Richard Furie
OBJECTIVE: In the present review, four new cases of bone marrow failure are presented and the potential contribution of systemic lupus erythematosus (SLE) is discussed. Furthermore, a comprehensive literature review of cases of autoimmune myelofibrosis (AIMF), aplastic anemia (AA), and paroxysmal nocturnal hemoglobinuria (PNH) with concurrent SLE aims to allow their direct comparison. Based on a clearer characterization of reported cases and our own experience, diagnostic and therapeutic strategies of these disorders in SLE are proposed based on lessons learned from the present and previous cases...
December 8, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29390349/sj%C3%A3-gren-s-syndrome-manifesting-as-clinicopathological-features-of-tafro-syndrome-a-case-report
#16
Shino Fujimoto, Hiroshi Kawabata, Nozomu Kurose, Haruka Kawanami-Iwao, Tomoyuki Sakai, Takafumi Kawanami, Yoshimasa Fujita, Toshihiro Fukushima, Yasufumi Masaki
RATIONALE: TAFRO syndrome is a newly proposed disorder that manifests as thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunction, and organomegaly. In this report, we describe the development of severe TAFRO syndrome-like systemic symptoms during the clinical course of juvenile-onset Sjögren's syndrome in a 32-year-old woman. PATIENT CONCERNS: The patient was admitted due to dyspnea, fever, polyarthralgia, and generalized edema. She had been diagnosed with Sjögren's syndrome at the age of 14 years, based on histopathological examination of a biopsy of the minor salivary glands and the development of Raynaud's phenomenon, with no follow-up treatment required...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29388258/allogeneic-hematopoietic-stem-cell-transplant-overcomes-the-adverse-survival-effect-of-very-high-risk-and-unfavorable-karyotype-in-myelofibrosis
#17
Ayalew Tefferi, Daniel K Partain, Jeanne M Palmer, James L Slack, Vivek Roy, William J Hogan, Mark L Litzow, Rhett P Ketterling, Mrinal M Patnaik
The prognostic importance of genetic information in primary myelofibrosis (PMF) was recently highlighted in a study of over 1,000 cytogenetically-annotated patients; 5-year survival rates were 8% for very high risk (VHR), 27% "unfavorable" and 45% "favorable" karyotype. The current study addresses the practice-relevant question of whether or not allogeneic hematopoietic stem cell transplant (HCT) can overcome the detrimental survival effect of VHR or unfavorable karyotype. The study included 67 patients with PMF or secondary MF who received HCT at the Mayo Clinic and in whom pre-transplant cytogenetic information was available...
February 1, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29383224/haploidentical-hematopoietic-stem-cell-transplantation-in-a-myelofibrosis-patient-with-primary-graft-failure
#18
Cristina Tecchio, Angelo Andreini, Claudio Costantini, Alberto Zamò, Donata de Sabata, Fiorenza Aprili, Roberta Galavotti, Emanuele Guardalben, Fabio Benedetti
No abstract text is available yet for this article.
December 22, 2017: Hematology Reports
https://www.readbyqxmd.com/read/29377227/lymphoproliferative-disorders-in-patients-with-chronic-myeloproliferative-neoplasms-a-systematic-review
#19
Monia Marchetti, Alessandra Carobbio, Enrica Capitoni, Tiziano Barbui
Patients with a Ph-negative myeloproliferative neoplasm (MPN) may harbor or develop lymphoproliferative disorders (LPD), however, the clinical and molecular determinants of MPN and LPD co-occurrence are still uncertain. In order to systematically pool the available knowledge, we conducted a scoping review of literature published since January 2005 and analyzed single-patient clinical data from 50 papers reporting 214 individuals harboring both MPN and LPD. Patients had been diagnosed essential thrombocythemia (44%), polycythemia vera (29%) or myelofibrosis (23%) at a median age of 67 years (26-94): half of them incurred a LPD after a median of 72 months from MPN diagnosis, while in 20% the LPD diagnosis was antecedent or synchronous...
January 27, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29372410/assessing-serum-albumin-concentration-lymphocyte-count-and-prognostic-nutritional-index-might-improve-prognostication-in-patients-with-myelofibrosis
#20
Marko Lucijanic, Ivo Veletic, Dario Rahelic, Vlatko Pejsa, David Cicic, Marko Skelin, Ana Livun, Katarina Marija Tupek, Tajana Stoos-Veic, Tomo Lucijanic, Ana Maglicic, Rajko Kusec
BACKGROUND: Primary and secondary myelofibrosis (PMF and SMF) are malignant diseases of hematopoietic stem cell characterized by the neoplastic myeloproliferation and a strong inflammatory milieu. The prognostic nutritional index (PNI) integrates information on albumin and absolute lymphocyte count (ALC) and reflects the inflammatory, nutritional and immune status of a patient. The clinical and prognostic significance of albumin, ALC and PNI in patients with myelofibrosis has not been previously investigated...
January 25, 2018: Wiener Klinische Wochenschrift
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